, Volume 22, Issue 10, pp 1231-1236
Date: 14 Mar 2006

Acute necrotizing encephalopathy: combined therapy and favorable outcome in a new case

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Abstract

Background

Acute necrotizing encephalopathy (ANE) is a rare disease characterized by multiple, symmetrical brain lesions, affecting thalami, brainstem tegmentum, and cerebellar medulla; more inconstantly, other structures are involved, i.e., internal capsules, posterolateral putamen, and deep periventricular white matter.

Features

The clinical picture consists of rapidly deteriorating acute monophasic encephalopathy preceded by prodromal febrile illness; the symptoms include hyperpyrexia, convulsions, recurrent vomiting, and coma within 24 h.

Prognosis

The outcome is usually poor and approximately 70% of the patients die within a few days from the onset of fever. There is no specific therapy for ANE but, in some patients, the clinical status improved with steroid treatment.